Myeloid sarcoma (MS) is a localized tumor composed of premature precursors of granulocytic cells, which may occur in any organ. and most commonly involves the soft tissue and musculoskeletal system. This malignancy may occur in the presence or absence of hematological disorders. Primary MS involving the central nervous system (CNS-MS) is rare, and has only been described in a small number of isolated case reports. The diagnosis of CNS-MS is challenging and strategies for its management are undefined. The present study describes 4 cases of CNS-MS. The hematological indices at admission were normal and all patients presented with location-associated nonspecific symptoms. In magnetic resonance imaging scans, the tumors appeared isointense on T1-weighted and T2-weighted images, with marked enhancement following contrast agent administration. Only 1 patient progressed to acute myeloblastic leukemia (AML), with this occurring 3 weeks following histological diagnosis. During the follow-up period, the patient with AML succumbed to the disease, local recurrence was noted in another patient for which a second surgery was requested and no progression was observed in the remaining 2 patients. CNS-MS has unique radiological characteristics and, due to its diffuse tissue infiltration, gross total resection is challenging. It is important for clinicians to be aware of potential hematological disorders in patients with CNS-MS. A combined surgical and chemotherapeutic strategy may be able to provide long-term control of this malignancy.